British Journal of Ophthalmology, 1992, 76, 183-185
Orbital paraganglioma: case report and review of the literature M M Bednar, T D Trainer, P A Aitken, R Grenko, R Dorwart, J Duckworth, C E Gross, W W Pendlebury
Abstract Paragangliomas ofthe orbit are extremely rare. A case of an orbital paraganglioma, including the first magnetic resonance imaging description of this tumour is described here. The patient underwent surgery with gross total removal of the tumour and relief of his initial chief complaint of visual blurring. The differential diagnosis and therapeutic options for the management of this tumour are discussed.
Paragangliomas are neoplastic growths of the non-chromaffin paraganglionic tissue which may arise in the abdomen, thorax, or head and neck region. Though paragangliomas of the head and neck are not uncommon they are rarely found in the orbit, where the site of origin is thought to be the ciliary nerve.1 Indeed only 27 cases have been reported in the literature. 1-23 The present report gives details of such a case and provides the first compilation of all previously described orbital paragangliomas.
Division of Neurosurgery, University of Vermont, Burlington, VT, USA M M Bednar J Duckworth C E Gross Division of
Ophthalmology, University of Vermont, Burlington, VT, USA
Figure 2 Nests of epithelioid cells in 'zellballen' pattern, characteristic ofparaganglioma (haematoxylin and eosin, x400).
Computed tomography (CT) of the orbits revealed a homogeneous mass lesion of 2 cm separate from and superolateral to the optic nerve. Prominent orbital veins were identified
The patient underwent a right lateral orbitotomy with visualisation of a firm, well circumCase history scribed mass with an adherent vascular capsule. The patient is a 55-year-old man who presented Frozen section examination suggested the diagto the Medical Center Hospital of Vermont with nosis of a meningioma. Therefore the operation intermittent visual blurring during the preced- was terminated after partial internal debulking ing 6 months. Physical examination revealed of the mass with a plan for postoperative radiaslight hypertropia and exotropia of the right eye. tion therapy.24 Vision was 20/25 OS and 20/20 OD. Pupils were Permanent microscopic sections of the tumour equal and reactive. Spontaneous venous pulsa- revealed nests of polygonal or spindle cells tions were absent on the right and normal venous surrounded by a delicate fibrovascular stroma forming the so-called 'zellballen pattern' (Figs 2 pulsations were present on the left. The visual field on the right showed concentric and 3). The tumour cells were moderate in size constriction of isopters and a small superior and round to polygonal in shape. The nuclei defect at 400 out. Colour vision was noted as 10/ were oval, slightly hyperchromatic, and 14-12/14. A right afferent pupillary defect of pleomorphic. Mitoses were rare. Immunohistoabout 1 2 log units was noted. chemical staining revealed focal positivity of the
P A Aitken
Department of Pathology, University of Vermont, Burlington, VT, USA T D Trainer R Grenko W W Pendlebury Department of Radiology, University of Vermont, Burlington, VT, USA R Dorwart Correspondence to: C E Gross, MD, Department of Surgery, University of Vermont, Burlington, VT 05401, USA. Accepted for publication 20June 1991
Figure I Enhanced axial CT scan of the orbit. There is a 1 5 cm x 2-0 cm well defined homogeneously enhancing mass that lies above the optic nerve in the apex of the muscle cone.
Figure 3 Reticulin stain outlining nests of cells in paraganglioma (reticulin, x200).
Bednar, Trainer, Ailken, Grenko, Dorwart, Duckworth, Gross, Pendlebury
ntion of the
tumour mass, in that the salt-andpepper appearance of the matrix is strongly I' ...,. suggestive of the diagnosis of paraganglioma.25 Sr d.r;, Prominent vessels might occur in association with other intraconal orbital masses, especially cavernous haemangioma, although this has not yet been described in reported cases.27-29 Intra'3 'p~~~~~~~~~~~~~~~~~~~r orbital meningiomas usually do not have grossly enlarged vessels that can be demonstrated by CT or MRI. Optic nerve glioma would not have a salt j.9 - and pepper matrix or prominent vascularity. Intraorbital metastases are usually not suffici*'.2 i +:ently ~ hypervascular to have grossly enlarged XS V vessels associated with them.30 -*"T While surgery is the treatment of choice for paragangliomas radiation therapy for unresecttumour cells for neuron specific enolase and able or incompletely resectable tumours, chromogranin. Ultrastructural examination unencapsulated tumours, and for recurrence has revealed occasional tumour cell,s containing been generally recommended.3' Tumour regression has been demonstrated using this proscarce to many neurosecretory gran ules (Fig 4). Six months postoperatively, a maignetic reson- cedure'0 though at least one case report failed to ance (MR) scan (Fig 5) of the ri«ght orbit was note any effect of postoperative radiotherapy obtained and demonstrated a 2-0 c-m by 1-8 cm (5000 cGy) on tumour size.2 However there was intraconal mass that displaced th(e optic nerve no discussion regarding the effect of radiation medially and inferiorly. The typic al prominent therapy on the patient's symptomatology. vessels and 'salt-and-pepper' appearance of the In summary this report reviews the pertinent MR image as described for paragPangliomas of literature to date and presents the pathological more typical locations were presentt.25 Because of and radiological appearance of an orbital pararesidual tumour and a recurrence oif his diplopia, ganglioma including the first MR description to the patient underwent a right Ifronto-orbital our knowledge. Although rare in this location a craniotomy as described by Jane et al2" with paraganglioma should be considered in the removal ofthe right orbital apex ma 5SS. Pathology differential diagnosis of a vascular orbital mass. was again consistent with the diaginosis of para- We acknowledge the referral of this patient by Dr Richard L ganglioma. The procedure was tern ninated when Dallow and the illustrations provided by Rolf Sennhenn, COT, CRA. tumour-free margins were obtaineci. V
Figure 4 Dense core granules (arrow) within the cytoplasm oftumour cells (x 11500).
-x:lt,t ** @
Discussion Paraganglioma of the orbit is a rare tumour with 27 such cases reported in the literatture. 1-23 The pathological differential diagnosis of orbital paraganglioma includes meningioma, metastatic adenocarcinoma (especiially renal cell carcinoma) alveolar soft part sarc oma, aveolar rhabdomyosarcoma, and hemangiiopericytoma. Ultrastructural demonstration of n eurosecretory granules in the chief cells of parn aganglioma is diagnostic.
The MR images allowed further characterisa-
5 6 7 8
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Figure S A, B Axial and coronal MR scans of the orbits demonstrate a well defined tumour mass in the muscle cone, sitting above and lateral to the right optic nerve (A; TR 2500 ms, TE 30 ms). Punctate and serpiginous structures with
flow voids represent prominent arteries and veins associated with the tumour (A). Note also the lateral orbitectomy defect in A and B. Coronal TI-weighted spin echo image (B; TR 600 ms, TE 20 ms) confirms intraconal location of the mass.
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